Immunoglobulin G4-associated autoimmune hepatitis with peripheral blood eosinophilia: a case report
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CASE REPORT
Immunoglobulin G4-associated autoimmune hepatitis with peripheral blood eosinophilia: a case report Arunchai Chang1 , Cheep Charoenlap2 , Keerati Akarapatima1 , Attapon Rattanasupar1 and Varayu Prachayakul3*
Abstract Background: Immunoglobulin G4 (IgG4) associated autoimmune hepatitis (AIH) has been recognized as a type of autoimmune disease that responds to corticosteroid. The diagnosis is based on elevation of the serum IgG4 level, abundance of IgG4 enhanced plasma cell infiltration in the portal region of the liver, and satisfaction of the criteria for “definite AIH” under the revised International Autoimmune Hepatitis Group (IAIHG) scoring system. However, the clinical course of the disease is unclear. Case presentation: A 65-year-old man with jaundice and peripheral blood eosinophilia.His IAIHG and simplified score was compatible with definite AIH and his IgG4 level was elevated. Magnetic resonance imaging did not reveal abnormalities in the hepatobiliary system or pancreas. A liver biopsy revealed interface hepatitis with IgG4 positive plasma cell infiltration in the portal region, without evidence of bile duct injury. He responded to 4-week period of induction prednisolone therapy and had no recurring symptoms under maintenance therapy of 5 mg prednisolone during the 3-year follow up. Conclusions: This was a rare case that demonstrated an association between IgG4 associated AIH and the presence of peripheral blood eosinophilia. Keywords: Immunoglobulin G4 associated autoimmune hepatitis, Autoimmune hepatitis, Immunoglobulin G4 related disease, Eosinophilia Background Autoimmune hepatitis (AIH) is an inflammatory liver disease characterized by chronic inflammation of the liver,positivity for autoantibodies, increased immunoglobulin level, and histological evidence of interface hepatitis and lymphoplasmacytic infiltration [1]. In contrast, immunoglobulin G4 (IgG4)-related disease is a chronic, relapsing, systemic, fibro-inflammatory syndrome of *Correspondence: [email protected] 3 Siriraj Gastrointestinal Endoscopy Center, Division of Gastroenterology, Department of Internal Medicine, Siriraj Hospital, Faculty of Medicine, Mahidol University, Bangkok 10700, Thailand Full list of author information is available at the end of the article
presumed autoimmune etiology with high blood levels of IgG4, IgG, and IgE [2]. The association between high serum IgG4 level and high peripheral eosinophil count has been proved; peripheral blood and tissue eosinophilia have been observed in some IgG4-related-disease patients [3]. Recently, AIH with an elevated serum IgG4 concentration and an abundance of IgG4-positive plasma cell infiltration in the liver was proposed to be termed “IgG4 associated AIH” and regarded as a subtype of IgG4-related disease [4]. However, there are few reported cases of IgG4 associated AIH (IgG4-AIH), and the clinical course of this disease remains poorly understood. Here, we report an interesting case of a patient with
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